Launois Syndrome

Acromegalic Gigantism

Launois syndrome (acromegalic gigantism) is typically caused by a pituitary tumor that secretes excess growth hormone. This condition results in the enlargement of skeletal and soft tissue, leading to characteristic gigantism and acromegaly.

Launois syndrome (LS), also known as acromegalic gigantism or pituitary gigantism, stems from the excessive production of growth hormone (GH) by the pituitary gland in the context of pituitary adenoma or hyperplasia, which sits in an enlarged sella turcica [1] [2]. At least 50% of LS cases are genetic in origin [3]. LS can occur as a mutation (for example in aryl hydrocarbon receptor interacting protein (AIP) gene), X-linked, familial or sporadic disorder.

There are two main phenotypic changes, namely gigantism, and acromegaly. Acromegaly typically occurs in adulthood, after the closure of the growth plates and sometimes in older children [4]. Gigantism typifies childhood onset of disease, when epiphyseal plates have not closed, leading to excessive growth of bones and thus an abnormallytall stature. Individuals with gigantism may experience less of the typical acromegaly features.

Acromegaly is insidious in onset, often occurring over years. In such cases, tumors are slow growing, leading to the average presentation in the fifth decade. Its clinical manifestations are a result of both skeletal and soft tissue growth, and these characteristically entail enlargement of the jaw, lips, nose and internal organs, for example, goiter. Furthermore, there is a growth of feet and hands, excessive sweating, increased body odor, arthropathy, joint stiffness and pain, and osteoarthritis-like features [6] [7].

Its clinical manifestations are a result of both skeletal and soft tissue growth, and these characteristically entail enlargement of the jaw, lips, nose and internal organs, for example, goiter.[symptoma.com]

Differential diagnosis should include the various lesions of the subcutaneous cellular tissue, such as lipomas, angiodysplasias, neurofibromas, sarcomas, goiters, sialoadenitis, obesity and lymphatic tumors.[rbcp.org.br]

Also in our patient, a subtotal resection of the thyroid was done in 1991 due to a nodular goiter and thereafter thyroxin was substituted. However, there was no hypothyrosis.[dercums_data.tripod.com]

The syndrome is often associated with features of metabolic syndrome such as diabetes mellitus, hyperuricemia, hyperlipidemia and hypertension and is associated with polyneuropathy which is an integral part of the disease.[ncbi.nlm.nih.gov]

Due to the massive accumulation of adipose tissue, the patients frequently have features of the metabolic syndrome as hypertension, impaired fasting glucose or diabetes mellitus, hyperuricemia or hyperlipidemia.[ncbi.nlm.nih.gov]

The patient was treated for hypertension and healthier lifestyle, and diet methods were suggested to improve the metabolic syndrome. The patient was subsequently referred to a plastic surgeon for liposuction of inguinal lipoma.[ncbi.nlm.nih.gov]

There is delayed puberty, muscleweakness, headache, perspiration; joint pain. At approximately 18 to 20 years of age abnormal growth starts and continues until 27 to 30 years of age. Possible, mental retardation, cephalgia, and visual defects.[whonamedit.com]

Symptoms related to the compressive effects of the tumor include headaches, visual disturbances such as bitemporal hemianopsia, and signs of hypopituitarism.[symptoma.com]

There is delayed puberty, muscle weakness, headache, perspiration; joint pain. At approximately 18 to 20 years of age abnormal growth starts and continues until 27 to 30 years of age. Possible, mental retardation, cephalgia, and visual defects.[whonamedit.com]

The peripheralneuropathy has often been laid to alcoholism, but the neuropathy is likely an integral part of the syndrome. This disorder affects mainly men and is more frequent in the Mediterranean area.[medicinenet.com]

MSL is associated with increased alcohol intake in up to 95% of cases and patients have also been known to suffer from other comorbidities such as peripheralneuropathy (80% of patients), macrocytic anemia, alcoholic fatty liver or cirrhosis, hyperuricemia[clinmedjournals.org]

Diagnosis of Launois syndrome relies on clinical evaluation and biochemical testing. Because of its slow progression, diagnosis can be made up to 10 years after disease onset. Biochemical parameters that are typically measured include:

Growth hormone: GH levels will be elevated. In some cases, the gonadotropin releasing hormone may also be overabundant, in instances where the secreting tumor is not located in the pituitary gland. GH measurements may be done after an oral glucose tolerance test (OGTT), where positive findings are GH in excess of 1ng/ml.

Insulin-like growth factor I: This will also be raised [8].

Prolactin: High prolactin levels are often reported.

Radiography of the skull and limbs is one of the imaging modalities carried out. Both computerized tomography (CT) and magnetic resonance imaging (MRI) scanning are useful in visualizing the tumor, however, MRI is more sensitive.

Screening for possible complications, such as diabetes and cardiovascular disease may involve specific tests such hemoglobin A1c (HbA1c), echocardiography and electrocardiography.

Launois syndrome (LS), also known as acromegalic gigantism or pituitary gigantism, stems from the excessive production of growth hormone (GH) by the pituitary gland in the context of pituitary adenoma or hyperplasia, which sits in an enlargedsella turcica[symptoma.com]

The current treatment of the disease is described based on the authors' experience. Four cases treated in our department are retrospectively reviewed regarding comorbidities and type of surgery performed.[ncbi.nlm.nih.gov]

Therapeutic options are pharmacological treatment with salbutamol and surgical procedures such as lipectomy or liposuction.[ncbi.nlm.nih.gov]

Common regions involved include the head and neck area, shoulder, and upper arms Complete excision by surgery or liposuction is the preferred treatment option for Madelung Disease presenting significant symptoms.[dovemed.com]

Pages: 4 Number of Figures: 3 Number of Tables: 1 ISSN: 1018-8665 (Print) eISSN: 1421-9832 (Online) For additional information: Abstract Benign symmetric lipomatosis, also known as Madelung’s disease or Launois-Bensaude syndrome, is a rare disease, the etiology[karger.com]

Etiology and Pathophysiology The etiology and pathogenesis of lipomas is unclear. A pathogenetic link between soft tissue trauma and the formation of lipomas has been suggested.[unboundmedicine.com]

Must be differentiated from other tumors, particularly liposarcomas because treatment protocols differ Epidemiology Lipomas can occur at any age but most commonly occur in middle-aged adults, typically in the 40- to 60-year-old age group, and more likely[unboundmedicine.com]

[…] an estimated incidence rate of 1 in 25,000, and affects males up to 30 times more frequently than females. [2] Cause [ edit ] The cause of the disease remains unknown, but its incidence strongly correlates with alcohol abuse ; abstinence from alcohol prevents[en.wikipedia.org]

Article

de Herder WW. Acromegaly and gigantism in the medical literature.Case descriptions in the era before and the early years after the initial publication of Pierre Marie (1886). Pituitary. 2009;12(3):236-244.

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